thioguanine-anhydrous and Preleukemia

A 9-month-old boy with known familial neurofibromatosis type I (NF-1) presented with a clinical and laboratory picture suggestive of juvenile chronic myelomonocytic leukemia (JCMMoL). Chromosomal studies obtained from the bone marrow indicated, however, that he had monosomy 7 syndrome. We believe this is the first reported case of monosomy 7 syndrome in a child with NF in the United States, and that this case complements a recent report of two cases of NF, JCMMoL, and monosomy 7 in Japanese children. Since monosomy 7 syndrome is very difficult to differentiate from JCMMoL or acute nonlymphocytic leukemia (ANLL) unless appropriate chromosomal studies are obtained, we believe it is possible that monosomy 7 may occur with increased frequency in patients with NF-1. Monosomy 7 syndrome might therefore be a significant cause of the known association between NF-1 and nonlymphoid leukemia.

Topics: Antineoplastic Combined Chemotherapy Protocols; Bone Marrow Transplantation; Chromosomes, Human, Pair 7; Combined Modality Therapy; Cytarabine; Daunorubicin; Dexamethasone; Diagnosis, Differential; Etoposide; Humans; Infant; Leukemia, Myelomonocytic, Chronic; Male; Monocytes; Monosomy; Neurofibromatosis 1; Pedigree; Preleukemia; Syndrome; Thioguanine

Topics: Antineoplastic Combined Chemotherapy Protocols; Bone Marrow Transplantation; Child; Child, Preschool; Combined Modality Therapy; Cross-Sectional Studies; Cytarabine; Down Syndrome; Doxorubicin; Humans; Iceland; Infant; Infant, Newborn; Leukemia, Myeloid, Acute; Leukemia, Myelomonocytic, Acute; Life Tables; Multicenter Studies as Topic; Preleukemia; Remission Induction; Scandinavian and Nordic Countries; Survival Rate; Thioguanine

These advances in chemotherapy and supportive care of the myeloid leukaemias offer substantially improved prospects for the future. At present the intensive treatment of acute and chronic myeloid leukaemia requires the resources of a specialist unit and as many patients as possible should now be referred for diagnostic classification and remission induction. Thereafter, courses of maintenance chemotherapy can be supervised jointly by the referring local physician and the specialist centre. This collaborative approach is to the mutual advantage of the patient, his local clinician, and the specialist centre.

Topics: Adult; Aged; Antibiotics, Antineoplastic; Antineoplastic Agents; Busulfan; Child; Clinical Trials as Topic; Cytarabine; Daunorubicin; Drug Administration Schedule; Drug Therapy, Combination; Humans; Leukemia, Myeloid; Leukemia, Myeloid, Acute; Preleukemia; Remission, Spontaneous; Thioguanine

Despite consolidation and/or maintenance chemotherapy most patients with newly diagnosed acute myelogenous leukemia relapse such that only 20-30% survive free of recurrence at five years. To evaluate the long-term effects of dose-intensive consolidation, we analysed 123 consecutive patients, age 16 to 84 (median 48 years), who received high-dose cytarabine-based consolidation chemotherapy. After a median follow-up of 88 months (range 26 to 126 months), 38 patients remain alive, with 26 in continued remission from 45 to 126+ months. Median remission duration for all eligible patients is 14 months (range 1.3 to 126 months) and actuarial leukemia-free survival at five years is 24 +/- 8%. Median survival from remission is 24 months (range 1.3 to 126 months) and actuarial survival from remission is 31 +/- 9%. Eighty-two patients (67%) have relapsed with an actuarial risk of relapse of 71 +/- 9% at five years. Adverse prognostic factors were age over 45 and male gender. When compared to historical controls (P = 0.02), dose-intensive consolidation produced improved leukemia-free survival for patients age < 45, but compliance and enhanced toxicity in the older age groups may limit further dose intensification.

Topics: Acute Disease; Adolescent; Adult; Age Factors; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols; California; Cytarabine; Daunorubicin; Drug Administration Schedule; Female; Follow-Up Studies; Humans; Leukemia, Myeloid; Life Tables; Male; Middle Aged; Mitoxantrone; Preleukemia; Remission Induction; Survival Analysis; Thioguanine; Treatment Outcome

Topics: Adolescent; Adult; Aged; Antineoplastic Combined Chemotherapy Protocols; Cyclophosphamide; Cytarabine; Daunorubicin; Drug Evaluation; Female; Humans; Leukemia, Myeloid, Acute; Male; Middle Aged; Myelodysplastic Syndromes; Preleukemia; Remission Induction; Thioguanine

One hundred ninety-six patients with acute myelogenous leukemia (AML) were treated with intensive induction chemotherapy using similar daunorubicin/cytarabine/thioguanine regimens. Treatment results of 44 patients who had a documented preleukemic syndrome or cytopenia present for more than 2 months before developing over AML were compared with 152 patients with de novo AML. Eighteen (41%) patients with preleukemia evolving into AML achieved complete remission compared with 111 (73%) patients with de novo AML (P less than .01). Patients with preleukemia-AML had a significantly longer period to recovery of granulocytes. Multivariate analysis indicated that presence of a previous preleukemic syndrome and advancing age were independent poor prognostic indicators for achieving remission. For patients who achieved remission, disease-free survival and overall survival were also inferior for patients with previous preleukemia; disease-free survival was 17 +/- 17% at 3 years compared with 29 +/- 10% in patients with de novo AML (P = .02). These data indicate that intensive chemotherapy has limited efficacy in patients with AML following a preleukemic syndrome. Durable remissions may be achieved in some patients.

Topics: Age Factors; Anemia, Aplastic; Anemia, Refractory, with Excess of Blasts; Cytarabine; Daunorubicin; Humans; Leukemia, Myeloid, Acute; Multivariate Analysis; Neural Tube Defects; Preleukemia; Prognosis; Regression Analysis; Survival Rate; Thioguanine

A consecutive series of patients (1978-1981) comprising all patients with acute leukaemia from a population of 475000 inhabitants was reviewed. Thus, 94 patients were diagnosed as having acute leukaemia. No patients were lost from follow-up. The incidence figures of ALL and AML differed significantly from those of Sweden as a whole. 9 patients were less than 15 years old. The median age of adult patients was 64 years, 60.8% being greater than or equal to 60 years old. Of adult patients with AML, 20% had a preleukaemic history (chronic myeloproliferative disorders, myelodysplastic syndromes and others). None of 6 patients with leukaemia as a metamorphosis of a chronic myeloproliferative disorder achieved a complete remission. The overall remission rate of the remaining adult patients was 25%. Treated patients, 15-39 years old, with AML without any preleukaemic history, had a complete remission rate of 80% compared to 12% for patients greater than or equal to 60 years old with the same diagnosis. Of 60 patients with 'primary' AML, 14 were not treated, mainly because of advanced age and complicating diseases. Most of these patients died within a week of admission.

Topics: Adolescent; Adult; Age Factors; Aged; Cytarabine; Daunorubicin; Female; Humans; Leukemia, Lymphoid; Leukemia, Myeloid, Acute; Male; Middle Aged; Prednisolone; Preleukemia; Sweden; Thioguanine; Vincristine